A sixties year-old man with a history of systemic sclerosis was referred to our hospital because of elevated liver enzymes. Abdominal finding of images showed massive hepatic necrosis near the diaphragm. Elevated ALP, AMA positive, and histological findings as PBC and elevated ALT and histological interface hepatitis findings as AIH indicated the diagnosis as overlap syndrome. Even though this case had good response with corticosteroid and UDCA therapy, hepatic scar and deformation remained after 6 months later. Liver biopsy must be considered aggressively in case of patients with collagen diseases who had elevated liver enzymes.
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